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Comprehensive care and Sickle Cell Disease and Hemoglobinopathies

Comprehensive care and Sickle Cell Disease and Hemoglobinopathies. Roshni Kulkarni MD. Distinguished Hematology Consultant Division of Blood Disorders National Center on Birth Defects and Developmental Disabilities Centers for Disease Control and Prevention.

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Comprehensive care and Sickle Cell Disease and Hemoglobinopathies

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  1. Comprehensive care and Sickle Cell Disease and Hemoglobinopathies Roshni Kulkarni MD. Distinguished Hematology Consultant Division of Blood Disorders National Center on Birth Defects and Developmental Disabilities Centers for Disease Control and Prevention The findings and conclusions in this presentation are those of the author and do not necessarily represent the views of the Centers for Disease Control and Prevention

  2. SCD issues across life span Roshni Kulkarni M.D

  3. Impact of SCD on Public Health • Insufficient data on prevalence may hamper effective management (immigrant hematology) and resource allocation • Impact of SCD on Mortality and Morbidity • Disability from complications • Impact on QOL • Preventable organ damage • Economic impact/ cost of care • Loss of workforce • Hospitalizations, emergency room care, blood transfusions/blood safety • Effectiveness of programs - must be sensitive to cultural practices and appropriate for given social context (World Health Assembly 2006)

  4. Challenges in SCD • Lack access to specialty care - expensive emergency department and inpatient services • Only a small fraction take Hydroxyurea • Many patients on Medicaid • Not enough funding compared to other genetic disorders • Federal agencies need to pool resources to coordinate services by supporting network of centers (large and small) across USA • Comprehensive care not available nationwide • Few funded sickle cell centers that serve fraction of the population • Current research advances need to be widely disseminated

  5. What About Sickle Cell trait? • 2 million SCT (1in 12 AA, 1 in 100 Hispanic Americans). Emerging Hispanic/migrant population • Thrombosis1 (VTE) 2-foldrisk PE 4-fold risk • Renal disease: renal medullary carcinoma, hematuria, papillary necrosis • Exercise related sudden death • Pregnancy related complications • ↓odds of early preterm delivery and odds of multiple gestations. • fetal losses and placental abnormalities. Austin Blood. 2007, Tsaras. Am J Med, 2009110:908, 2007. 2. Bryant AS. Obstetrics & Gynecology, 2007

  6. Public Health & SCD:Reduce Death, Disability, Promote Health • Determine Incidence and Prevalence • Surveillance for : • Complications • Blood safety monitoring: pathogens, alloimmunization • Long term follow up of NBS & Screening of older/migrant population • Adult vaccinations • Gynecologic and reproductive issues • Rural/Urban outcomes

  7. Public Health & SCD (cont’d) • SCD specific QOL instrument : • Pain & Disability • Psychosocial and behavioral issues • Transition of care/ effectiveness • Health Education • Patient/provider/school education • Prevention message through Community based organizations • Public health laboratory • Transfusion transmitted disease • Genotyping on a mass scale: high throughput genotyping • Role of gender and race

  8. Health Promotion through Comprehensive care Comprehensive Care Centers Registry Surveillance Diagnosis:Disease burden Trends in health Emerging/existing risks Hemovigilance Surveillance maps Education and prevention messages

  9. Comprehensive care Health Systems Research Clinical and basic research Quality indicators Training program support Registry and Prospective Surveillance Comprehensive Care Centers Public Health Research New Drug Development Blood safety Education and prevention messages Access to specialty care and laboratory

  10. Comprehensive Care Model • Organized specialized care delivery • Integrated multi-disciplinary teams • Emphasis on • Early diagnosis • Prevention of complications • Reduction in disability • Improves survival and QOL • Education and training of patients/providers • Coordinated network of centers to improve care through public health and clinical / translational research (bench to trench) • Liaison with community organization (s)

  11. How dowe develop comprehensive hemophilia care in a country? “BUT”--We must first understand the Economics of Care! Evatt WFH

  12. Economics: The Primary Force in Available Care In countries with emerging economies, economic issues will be the force determining how far we can push our goals… In developed countries, only constant pressure prevents reduction in services. We must understand these issues and use them to our advantage…

  13. Economic Reality • Care is expensive. • Cost of care beyond reach of individual. • Will rely on others to pay bill. • Will have to convince others to do so. • Government must be involved. • Government focuses on bottom line, economics and the most served for the least cost.

  14. Effect of Economic Capacityon Hemophilia Adulthood <$2,000 $2,000 – $10,000 >$10,000 Per Capita Gross National Product Evatt & Robillard., Haemophilia (2000)

  15. Effect of Care Programson Hemophilia Adulthood <$2,000 <$2,000 + HTC Per Capita Gross National Product Evatt & Robillard., Haemophilia (2000)

  16. Effect of Comprehensive Care • Life Expectancy 13-40 65 • Days in hospital 12-15 1 • Percent with Joint disease 70-80% <5% • Annual Visits to Clinic 12-30 0-6 • Unemployment 35-90% 15% Without Comprehensive Care With Comprehensive Care

  17. Components of Comprehensive care team • Core team: • Medical Director- a hematologist • Coordinator- Nurse/program • Psychosocial professional • Secretary • Extended team • Pediatrician/internist/Family practitioner • Genetic counselor /Ob-Gyn • Nutritionist /Dental/ other subspecialist – Pulmonologist etc • Must have field or outreach services

  18. Registry and SurveillanceWorld Federation of Hemophilia 2005. • Prevalence • Determine current and future needs of the community • Empowering advocacy groups, physicians • Resource allocation: set priorities of health outcomes • ↓ Hospitalizations, Improved QOL

  19. Registry and SurveillanceEvatt B. World Federation of Hemophilia 2005 Maintaining and Using a registry • Develop policy “ we will do….will help 300 people and cost $24,000” • Develop and manage resources: identify areas of need • Define changing/expanding populations needs: vaccination • Disease management, Disaster preparedness*

  20. Surveillance Aim: reduce incidence, morbidity and mortality • Definition: Systematic collection, analysis, interpretation, and dissemination of health data • Assist: planning, implementation, and evaluation of public health interventions and programs. • SCD surveillance (web based) • Complications across life span • Impact of risk factors, co-morbid conditions • Impact of newborn screening • Blood safety. Lab studies. • Quality of Life: cultural and social context/transition issues • Preconceptual care/Reproductive issues

  21. Components of a SCD Surveillance System • Comprehensive • Linking multiple data sources • Population-based • Longitudinal Design • Modular Design • Quality Assurance Measures • Web-based /Electronic Reporting • Unified and Standardized Laboratory Testing • Specimen Collection and Blood Safety Monitoring

  22. Identify data sources for indicators Grant A. DBD/NCBDDD/CDC

  23. Mapping • Geographic Information Systems (GIS) can be used to analyze data and create maps • Over 90% public health data have a geospatial component • Maps can be useful for: • Describing the distribution of patients • Planning of care • Resource planning • Outreach activities • Emergency /disaster preparedness

  24. Hospitals with Emergency Departments Hospitals with ED and patients Geographical Information SystemMcClellan A. Division of Blood Disorders. NCBDDD.CDC

  25. McClellan Ann. CDC DBD GIS expert

  26. Role of Patient Advocacy • Do not become complacent! • Must understand health care needs and set clear realistic goals to achieve them • Must work hand in hand with physicians to speak as one voice • Must understand the political system • Must work closely with government officials

  27. SCD and Hemophilia

  28. So What Can One Do? • In Hemophilia- population expanded to include thrombosis, Women and other groups • In SCD can partner with hemoglobinopathies • Ultimate solution for viability………

  29. Hematology Specialty Care: Future Blood Disorders Center Model Bleeding Red cell/Hbpathies Iron Clotting Lifespan issues Complications Hemovigilance Management Research Disparities White cell BM failure Advocacy groups S Shurin & R Kulkarni

  30. Blood Disorders Center Model Guam Coordinating centers Puerto Rico

  31. International Blood Disorder Centers

  32. Acknowledgements • Hani Atrash • Mike Soucie • Althea Grant • Bruce Evatt • Melissa Creary • Ann McClellan • Christopher Parker • Susan Shurin NHLBI

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